Transthoracic echocardiography and 6-minute walk test in Kuwaiti sickle cell disease patients

The aim of this study was to investigate cardiac abnormalities in Kuwaiti sickle cell disease [SCD] patients using markers such as tricuspid regurgitant jet velocity [TRJV], pulmonary artery systolic pressure [PASP], and the 6-minute walk [6MW] test and correlate these findings with clinical, hematological, and biochemical parameters. Seventy-three patients with SCD and 70 matched controls were studied. The cardiac status was investigated using transthoracic echocardiography in 57 patients; the 6MW test was carried out in patients and controls. Complete blood counts and hemolytic parameters were assessed. Reticulocytes, bilirubin, and lactate dehydrogenase were significantly higher [p < 0.0001] in patients, while hemoglobin [Hb] and haptoglobin were lower [p < 0.0001] than in controls. The mean fetal Hb among patients was 15.85 +/- 8.7%. Of the 57 patients, 14 [24.5%] and 15 [26%] had mild tricuspid and mitral regurgitation, respectively. The mean ejection fraction, TRJV, and PASP were 63.9 +/- 6.3%, 1.7 +/- 0.5 m/s, and 23.0 +/- 7.3 mm Hg, respectively. Three [5.2%] patients had mildly raised TRJV [2.6-2.97 m/s, normal range <2.5 m/s] while 8 [14%] had high PASP [mean 35.3 +/- 5.1 mm Hg, normal range <30 mm Hg]. Hb, hematocrit, and reticulocytes were different [p = 0.010, p = 0.006, and p = 0.011, respectively] between patients with normal and high PASP. All 3 patients who had a high TRJV had a high PASP, and 2 of these patients died during follow-up. The systolic and diastolic blood pressure, oxygen saturation before and after the 6MW test, and distance walked were lower [p = 0.006, p = 0.000, p = 0.002, p = 0.000, and p = 0.000, respectively] in patients compared to controls. Raised PASP was common in Kuwaiti SCD patients while raised TRJV was not

Acute splenic infarction following air travel in a child with Hb SD disease

The relative hypoxia, dehydration and immobility associated with air travel predispose sickle cell disease patients to acute splenic crises; however we are not aware of previous reports involving children with Hb SD. Clinical Presentation: We report on a previously healthy 2-year-old Kuwaiti girl who developed a sudden onset of severe abdominal pain while traveling on a commercial jet aircraft. This was followed by vomiting and fever. On examination in the emergency room she was very irritable with abdominal tenderness maximal over the left hypochondrium. The spleen was palpable 7 cm below the costal margin, and abdominal ultrasonography and CAT scan confirmed a diagnosis of acute splenic infarction. Hematological investigations showed that the patient was a compound heterozygote for Hbs S and D. Intervention: The patient was managed with intravenous fluids and analgesics and made a full recovery. Acute splenic infarction should be considered in the differential diagnosis of a sudden onset of abdominal pain associated with air travel in patients with Hb SD

Current trends in the management of sickle cell anemia

Although there has been enormous accumulation of knowledge about the molecular biology of sickle cell anemia, progress in clinical management has not been very impressive. Recently, however, the use of pharmacological agents, especially hydroxyurea, to manipulate HbF levels, bone marrow transplantation for selected cases and research into gene therapy have re-kindled hope for successful control and "cure" of the disease. The inherent variability in the clinical course of the disease, however, means that these new modalities of treatment are not appropriate for all patients. In this article the determinants of clinical variability and the available management options are reviewed especially as applicable to patients in the Arabian Peninsula